Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery
Williams Syndrome (WS) is a complex neurodevelopmental disorder associated with a mutation on chromosome 7. Patients with WS usually display dysmorphic facial and musculoskeletal features, congenital heart diseases, metabolic disturbances and cognitive impairment. Structural cardiovascular abnormali...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Case Reports in Anesthesiology |
| Online Access: | http://dx.doi.org/10.1155/2016/1371095 |
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| author | Federico Boncagni Luca Pecora Vasco Durazzi Francesco Ventrella |
| author_facet | Federico Boncagni Luca Pecora Vasco Durazzi Francesco Ventrella |
| author_sort | Federico Boncagni |
| collection | DOAJ |
| description | Williams Syndrome (WS) is a complex neurodevelopmental disorder associated with a mutation on chromosome 7. Patients with WS usually display dysmorphic facial and musculoskeletal features, congenital heart diseases, metabolic disturbances and cognitive impairment. Structural cardiovascular abnormalities are present in the majority of the children and may provide a substrate for perioperative Sudden Cardiac Death, as presented by several reports, something that creates a great challenge to the anesthetic conduct. We present the case of a 12-year old girl who required anesthetic care for surgical correction of an acquired kyphoscoliosis. Potential anesthesiological implications of WS are subsequently reviewed. |
| format | Article |
| id | doaj-art-4064db9ef52649f4bf249461aa9b68f0 |
| institution | Kabale University |
| issn | 2090-6382 2090-6390 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Anesthesiology |
| spelling | doaj-art-4064db9ef52649f4bf249461aa9b68f02025-08-20T03:55:36ZengWileyCase Reports in Anesthesiology2090-63822090-63902016-01-01201610.1155/2016/13710951371095Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine SurgeryFederico Boncagni0Luca Pecora1Vasco Durazzi2Francesco Ventrella3Clinica di Rianimazione Generale, Respiratoria e del Trauma Maggiore, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, ItalyAnestesia e Rianimazione dei Trapianti e della Chirurgia Maggiore, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, ItalyClinica di Neurologia, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, ItalyAnestesia e Rianimazione Pediatrica, Azienda Ospedaliero-Universitaria “Ospedali Riuniti Umberto I-G. M. Lancisi-G. Salesi”, 60126 Ancona, ItalyWilliams Syndrome (WS) is a complex neurodevelopmental disorder associated with a mutation on chromosome 7. Patients with WS usually display dysmorphic facial and musculoskeletal features, congenital heart diseases, metabolic disturbances and cognitive impairment. Structural cardiovascular abnormalities are present in the majority of the children and may provide a substrate for perioperative Sudden Cardiac Death, as presented by several reports, something that creates a great challenge to the anesthetic conduct. We present the case of a 12-year old girl who required anesthetic care for surgical correction of an acquired kyphoscoliosis. Potential anesthesiological implications of WS are subsequently reviewed.http://dx.doi.org/10.1155/2016/1371095 |
| spellingShingle | Federico Boncagni Luca Pecora Vasco Durazzi Francesco Ventrella Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery Case Reports in Anesthesiology |
| title | Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery |
| title_full | Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery |
| title_fullStr | Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery |
| title_full_unstemmed | Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery |
| title_short | Anesthesiological Management of a Patient with Williams Syndrome Undergoing Spine Surgery |
| title_sort | anesthesiological management of a patient with williams syndrome undergoing spine surgery |
| url | http://dx.doi.org/10.1155/2016/1371095 |
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